Early diagnosis of congenital tooth agenesis, increases the quality of treatment options and the final success of the treatment. However, in some clinical conditions, overlooking of this diagnosis causes late diagnose of these anomalies. Late diagnosis reduces the number of appropriate treatment, increases the cost of treatment and possible complications. In the diagnosis of congenital tooth agenesis, findings in the routine medical examination of the pediatric patient are beneficial. At this point, as well as pediatric dentists, pediatricians also play a great role in early diagnosis of these anomalies. The aim of this study is to draw attention to the pediatric dentist-pediatrician cooperation in early diagnosis of congenital tooth agenesis.

INTRODUCTION: Feeding with a syringe has been used as an alternative feeding method for preterm infants.
METHODS: This study was conducted at a level II neonatal intensive care unit (NICU) in the Zekai Tahir Burak Maternity Teaching Hospital in a comparative and descriptive clinical study pattern. The study was carried out with 47 preterm infants in a syringe fed group (SG) and 56 preterm infants in a bottle fed group (BG). Primary outcomes were time of transition from gavage feeding to full oral feeding, time of transition from tube to breastfeeding, and length of hospital stay. Secondary outcomes were body weight at discharge (g), daily body weight gain (g/days) and gastro-intestinal intolerance symptoms during the transition period.
RESULTS: Mean gestational ages were 29.82 ±2.03 vs. 28.18±1.56 weeks (p = 0.24) and mean birth weights were 1150.31±232.29 vs. 1016.87±186.64 g (p=0.72) in the SG and BG groups, respectively. 103 infants receiving gavage feeding with gestational ages ranging from 26 to 32 weeks were evaluated for full oral feeding start time. Syringe fed preterm infants had a mean of 40.45±19.50 days and bottle fed infants had a mean of 53.81±16.97 days (p>0.05). The time to transition to breastfeeding (42.54 ±21.21 days) and time to discharge (54.48 ±26.92 days) in the SG was significantly shorter compared to the BG (50.45 ±15.95, 67.21 ±22.07, respectively) (p<0.05).
DISCUSSION AND CONCLUSION: We found that preterm infants for whom feeding with a syringe was used as a reinforcement in addition to orogastric feeding switched to full breastfeeding in a shorter time compared to infants who were fed by bottle. From these results we recommend syringe feeding as a transitional method prior to breastfeeding for preterm infants during hospitalization.

INTRODUCTION: Despite improvements in diagnosis and treatment, infections are still major cause of morbidity and mortality in children with febrile neutropenia (FN). In these patients, due to inadequate inflammatory response and subtle clinical symptoms, to determine the source of infection can be challenging.Therefore, it is important to distinguish infections from other non-infectious causes, for both to choose appropriate antibiotic and to reduce the redundant antibiotic use.
METHODS: In this retrospective study, we aim to evaluate serial procalcitonin (PCT) levels for predicting bacteremia particularly caused by Gram-negative microorganism.
RESULTS: Among FN episodes caused by Gram-negative microorganism, the median level of second PCT sample obtained between 48 and 72 hours (PCT 2) was found to be significantly higher when compared to infections caused by Coagulase-negative Staphylococcus (CoNS) or culture-negative confirmed infections, P value was 0.003; however, fever onset PCT 1 and C-reactive protein (CRP) 1 values showed no significant difference (P>0.05). The area under curve (AUC) values demonstrated by ROC analysis for CRP 1, CRP 2, PCT 1, PCT 2 were 0.664, 0.748, 0.504 and 0.842, respectively.
DISCUSSION AND CONCLUSION: This study showed that initial PCT levels were not significantly correlate with culture-confirmed bacterial infection. Therefore, initial PCT values do not help the clinicians in terms of administering or postponing empirical antibiotics at the time of fever onset. However, third day PCT levels present as a good diagnostic marker due to a higher sensitivity and specificity when comparing them to the initial values. Determination of serial PCT may enhance the diagnostic value of PCT diagnostic marker in FN episodes caused by Gram-negative bacteria with a high sensitivity (87.5%). This study also demonstrated that PCT could be used to rule out bacterial infections particularly caused by Gram-negative bacteria.

INTRODUCTION: In many studies, relation of postural status and temporomandibular disorders (TMD) has been investigated, however there is no consensus on this regard. The aim of this study was to investigate the relation between postural irregularities (forward head posture-FHP and different shoulder levels-DSL) and (TMD) prevalence in different dentition stages.
METHODS: This study, which included children between 4 to 14 years of age attending the public schools in Ankara, was planned as a cross-sectional study. TMJ was examined intra and extraorally. After the examination, the relationship between TMD and body posture (FHP and DSL) was investigated. For the statistical analysis, Chi-Square Test and Fisher’s Exact Test were used with significance level of 0.05.
RESULTS: FHP and DSL were statistically related to the TMD (p<0.05). In primary dentition, there was no significant relation between the FHP and TMD, however, DSL were found to be significantly related to TMD (p<0.05). In mixed dentition, both of these parameters were statistically related to TMD (p<0.05). However, in the permanent dentition, there was no relation between the body posture and TMD.
DISCUSSION AND CONCLUSION: Since there is a strong correlation between postural irregularities and TMD especially in mixed dentition stage, TMJ and postural status of pediatric patients should be examined as possible as early stages of the life. In this regard, the awareness of pediatricians and pediatric dentists needs to be increased.

INTRODUCTION: Visceral leishmaniasis (VL) is a systemic infection that spreads hematogenously and affects the reticuloendothelial system by the infection of macrophages. VL occurs commonly in children, and only rarely in adults. VL should be considered in patients with prolonged high fever, hepatosplenomegaly, pancytopenia, weight loss, pallor and hypergammaglobulinemia.

METHODS: In this study, a total of 18 pediatric patients –9 (50%) males and 9 (50%) females– treated for VL at our clinic from January 2004 to July 2014 were analyzed retrospectively. Average time from symptom onset to hospital admission was 64 ± 21 days (range: 30-100 days). The mean age of patients was 88±40 months (range: 36-182 months).

RESULTS: The most common symptom at presentation was fever (88.9%). Other common symptoms were fatigue, chills, weight loss and anorexia. Physical examination revealed splenomegaly and hepatomegaly in all patients. Anemia (92.4%), leukopenia (78.7%) and thrombocytopenia (76.2%) were the most prominent laboratory abnormalities and 82.2% of the patients were pancytopenic on admission. Bone marrow smear was positive for leishmania in 100% of the patients. All patients received treatment with liposomal amphotericin B.

DISCUSSION AND CONCLUSION: In certain regions, increased humidity rates associated with construction of dams and irrigation canals may lead to structural changes in the ecological balance and thus cause an increase in the population of disease-spreading vectors. Additionally, recent migration from the middle-eastern region to western parts of the world due to regional civil wars may have contributed to the observed increase in the incidence of various diseases such as VL.

INTRODUCTION: Liver transplantation (LT) is performed for several indications in the pediatric population, including malignancy and acute hepatic failure among others. LT has become an important treatment alternative for metabolic diseases. In most pediatric transplant centers, metabolic liver disease is the second most common indication for liver transplantation after biliary atresia. Our studies aim is to compare survival outcomes with metabolic and other liver diseases in our transplant program.
METHODS: 189 patients who underwent liver transplantation between 1997 and 2015 due to metabolic diseases and acute or chronic liver failure were included in the study.
RESULTS: We enrolled 189 patients in our study. 54% (n = 102) male and 46% (n = 87) female patients were included in the study.In metabolic diseases group 56 patients were enrolled. Progressive familial intrahepatic cholestasis (PFIC) is the first among metabolic diseases resulting in LT; Wilson's disease was the second. Posttransplant immunosuppression was similar for both groups. Although there was no difference in both groups, regarding onset of the post-transplant complications, graft type, recipient age. Bilier and portal vein complications were most particularly defined in the group with nonmetabolic diseases. There was no significant difference in survival between the two groups.
DISCUSSION AND CONCLUSION: Liver transplantation is an important treatment option for acute hepatic failure and end-stage liver disease. In addition, liver transplantation is an alternative treatment option for some metabolic diseases.

INTRODUCTION: Cardiovascular involvement is common in patients with mucopolysaccharidoses (MPS). In this study, we investigated the effects of the markers involved in vascular endothelial injury pathogenesis (transforming growth factor β-TGF-β, interleukin-IL 6, IL 10, high sensitive C reactive protein-hs CRP, vascular endothelial growth factor-VEGF, N-terminal pro- Natriuretic peptide-NT-proBNP) and clinical, laboratory and echocardiographic findings of the patients
METHODS: A total of 37 patients (5 MPS I, 4 MPS II, 2 MPS IIIa, 4 MPS IIIb, 14 MPS IVa, 8 MPS VI) and 32 controls with similar age and sex were included in the study.
RESULTS: Corneal clouding was seen in 29(78%) patients. There were 23 (62%) patients with organomegaly, and 28 (75%) patients with hearing loss. When the groups were compared in terms of NT-proBNP, hsCRP, TGF-β, IL6, IL10 and VEGF levels, there was a statistically significant increase in the patient group for NT-proBNP and VEGF (p = 0.04, p = 0.03, respectively). The carotid intima media thickness was statistically significantly higher in the patient group (p <0.001). Left ventricular diastolic diameter was significantly higher in the patient group (p = 0.009), intraventricular septum thickness was significantly higher in the patient group (p <0.001). E/A ratio was significantly lower in the patient group (p <0.001).
DISCUSSION AND CONCLUSION: Cardiac involvement in MPS patients is a major cause of mortality and morbidity. It is thought that cytokines, proinflammatory markers are elevated in patients with vascular damage like other lysosomal diseases. There is a need for further studies to determine biomarkers for vascular involvement

INTRODUCTION: Soft tissue injuries which happened because of high heat, chemicals etc. called burns. Sleep is a complex behavior regulated by the interaction of anatomical and neurochemical areas in the central nervous system. After burn; patients are exposed to many stressors that cause sleep interruption and decreasing in sleep quality. The purpose of this study was to determine sleep characteristics, sleeplessness and sleep habits in pediatric burn patients.
METHODS: This descriptive study was carried out with 96 children who were 2-6year-old in Pediatric Surgery Department/Burn Unit for 2-7 days. For data collection; Demografic Data Collection Form, Sleep Problems Characteristics and Identification Survey which has been created by researchers are used. Data were collected by face to face interviews with mothers. Questions were asked to compare pre and post burn sleep quality.
RESULTS: Of the children, 53.1% were male and their mean age was 3.36±1.39 years. There was a significant differences between pre and post burn periods on children’s sleeping and waking hour, total and night sleep duration, total daily sleep duration, existence of sleep interruption and number of interruptions, trouble of falling asleep and sleep latency, being tired in the morning, forcing the children to wake up, duration of waking up.
DISCUSSION AND CONCLUSION: With the knowledge about the importance of sleep, attention should be paid to sleep of children that we are providing care. Supportive environmental regulations should be made to improve the quality of sleep in hospitals.

INTRODUCTION: We aimed to assess the prevalence of actinomycosis in tonsillectomy specimens and its clinical importance and management in our center.
METHODS: In this retrospective matched case-control study, patients who underwent tonsillectomy during 2010-2015 in Shahid Mostafa Khomeini Hospital, Tehran, were studied. Data regarding age, sex, indication for surgery (such as recurrent tonsillitis or sleep-disordered breathing), tonsil size, and length of disease before surgery were collected in both histopathologically positive (case) and negative (control) patients for actinomycosis.
RESULTS: From the 260 studied tonsillectomy specimens, actinomycosis was found in 20 (8.75%) and 60 Actinomycosis negative cases were chosen accidentally for control group. There was no statistically significant difference between the groups with respect to age, sex, length of disease before surgery, and incidence of actinomycosis in tonsillectomy due to recurrent tonsillitis or sleep-disordered breathing. However, the tonsil size was significantly greater in specimens positive for actinomycosis ( 8.65±1.5 versus 4.38±0.22, P<0.001).
DISCUSSION AND CONCLUSION: Actinomycosis in tonsillectomy specimens is an incidental histopathological finding and does not necessarily correlate with active disease; while it is associated with tonsillar hypertrophy and increases tonsil size.

INTRODUCTION: This study aims to examine how parents of children with cancer seek information through online communities, using netnography methods from qualitative study types.
METHODS: The study was carried out through two online communities where parents of children with cancer in Turkey communicate with each other. The data were obtained from the parents’ posts to online community platforms and covered the period between September 2 and November 2, 2017. The data were collected using the netnography method and analyzed with the principles of this method.
RESULTS: A total of 580 written posts (information sharing) were sent within the interval in which the study was conducted, and 208 of them, which were posted by 131 parents, were analyzed and coded. Accordingly, three themes were defined on the basis of codes as follows: “disease”, “treatment and side effects,” and “non-treatment” posts. The themes of “disease” and “treatment and side effects” included questions in which the parents were seeking information, whereas the theme of “non-treatment” mostly included posts in which the parents were seeking emotional support from other parents in the communities.
DISCUSSION AND CONCLUSION: The way in which parents of children with cancer seek information through online communities is mostly related to their questions and posts parallel to the cancer process. In this process, the parents were found to seek emotional support.

INTRODUCTION: Spinal muscular atrophy (SMA) is a devastating genetic disease in childhood and is caused by the absence of functional SMN protein, which leads to impairments of cytoskeleton, especially in neurons. Dysregulation of actin dynamics have been linked to SMA pathomechanisms, however involvement of altered microtubule dynamics is largely unknown. In this study, we investigated differentially expressed microtubule-related genes using in vitro and in vivo SMA model systems.
METHODS: By focusing microtubule-related genes, we re-analyzed publically available gene expression arrays, which were previously performed with induced pluripotent stem cell-derived motor neurons of SMA patient and spinal cords of SMA mice. We found altered expression of microtubule-associated protein 2 (MAP2), which was validated by real time RT-PCR using SMN knock-down NSC34 cell line and severe SMA mouse model.
RESULTS: We showed that expression of MAP2 gene was significantly upregulated in both expression arrays. Upregulation was also detected in brain and spinal cord tissues of severe SMA mice at different developmental stages.
DISCUSSION AND CONCLUSION: Our findings suggest that microtubule regulatory proteins may be altered in SMN depleted cells and further research are needed to elucidate contribution of dysregulated microtubule dynamics to SMA.

Fanconi Bickel Syndrome (FBS), also known as glycogen storage disease type XI, is a rare autosomal recessive disorder. This syndrome has many different identified mutations and it is rare to be diagnosed during the neonatal period. Our patient is a two-weeks old female newborn who was admitted to our hospital with fever and dehydration. Renal Fanconi Syndrome was diagnosed in the presence of polyuria, proteinuria, glycosuria, hyperchloremic metabolic acidosis with normal anion gap and positive urine anion gap, hypourisemia, hypophosphatemia and increased excretion of phosphorus in urine. A novel mutation, IVS8 homozigot g.24401-24406del6 in the GLUT2 gene was demonstrated by Sanger method. The same mutation was detected as heterozygote in her parents. Although most of the affected infants have a consanguineous parentage history in the literature, our patient was born to a non-consanguineous parents. The interest of our FBS case lies in the timing of diagnosis which happened in the patient’s first month of life as only a few cases of FBS are recognized in this period as well as having a novel mutation in the literature.

Intralobar pulmonary sequestration (ILS) is a rare congenital malformation, usually diagnosed later in childhood or adolescence. We report a case who presented with tachypnea and diagnosed at 2-months-old. Pulmonary sequestration (PS) usually managed by embolisation or surgical resection. Recently, preoperative embolization of aberrant arteries to minimize the risk of serious intraoperative haemorrhage has also been described. Our case was also successfully treated with embolization followed by a thoracoscopic resection.

Hereditary neuropathy with liability to pressure palsies (HNPP) characterized by repeated focal pressure neuropathies is rare in childhood. Here we present a 10-year-old girl admitted to our hospital with recurrent weakness in her foot and diagnosed as HNPP with a quick Multiplex Ligation-dependent Probe Amplification (MLPA) test showed PMP 22 deletion.

Fibrinous bronchitis, also known as plastic bronchitis or pseudomembranous bronchitis, usually manifests clinically with nonproductive cough and dyspnea. Prior to the expectoration of plaque formations, patients have a history of viral or other respiratory disease. Plastic bronchitis is an extremely rare and fatal complication associated with Fontan procedure. The condition is characterized by the formation of inspissated bronchial casts which may cause life-threatening airway obstruction. Although the pathogenesis of this condition remains unclear, it is believed to involve elevated pulmonary venous pressure, increased central venous pressure, and endobronchial lymphatic leakage.A 9-year-old male patient with Down syndrome underwent Fontan procedure 29 months earlier due to complete endocardial cushion defect and single ventricular physiology. The patient presented to the emergency department due to sudden-onset respiratory distress and fever (38.5°C). Bilateral diffuse sibilant rhonchi, secretory rales, and intercostal retractions were noted during pulmonary system examination. Posterior-anterior chest x-ray showed cardiomegaly (cardiothoracic ratio=0.6) and bilateral diffuse infiltration.While under treatment, the patient experienced sudden-onset cough with expectoration of rubbery sputum in the form of branching bronchi-shaped casts.Samples of the inspissated sputum were sent to the Pathology and Microbiology departments for examination. The pathology report indicated fibrinoid material composed of a small number of inflammatory cells and bacterial plaques. Normal bacterial flora was identified in microbiological culture.Clinical presentation can vary from mild clinical findings to life-threatening symptoms. As in our patient, the diagnosis is made clinically, based on expectoration of bronchial casts or their detection during bronchoscopy. As our patient responded well to medical treatment, we proceeded with clinical follow-up.
Plastic bronchitis is a very rare entity, and carries a poorer prognosis when it develops after congenital heart disease, as in our case.